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Abstract

Background: Takayasu’s disease is a rare chronic inflammatory disease of unknown etiology and affects the aorta and its main branches. Little is known about the long-term prognosis of Takayasu’s disease despite of younger peak age of the disease onset. Methods and results: We conducted extended follow-up of the Ishikawa’s original cohort which included consecutive 120 patients with Takayasu’s disease in our institution. Median clinical follow up period was 20.3 years (IQR 13.2-30). The overall mortality at 5, 10, and 20 years was 91.6%, 88.8%, and 84.4% after the diagnosis, respectively. After adjusting for potential confounding factors, major complications (Takayasu’s retinopathy, hypertension, aortic regurgitation, and aneurysm) and progressive course (marked by the deveropement of progressively severe symptoms after a period of years since onset) were independently associated with poor long term prognosis (major complications: adjusted HR 5.2 95%C.I 1.8-18.5, progressive course: adjusted HR 2.7 95%C.I 1.1-7.4).

Conclusion: The long-term outcome for patients with Takayasu’s disease seems best predicted two major prognostic factors. Patients with those poor prognostic indicators may require more aggressive management and close follow up not only acute phase but also chronic phase.